History

With the increasing use of prenatal ultrasonography as well as improvement in technology and skill, most cases of congenital cystic adenomatoid malformation (CCAM) are prenatally diagnosed. Prenatally diagnosed lesions can present with various symptoms.

This is the presenting symptom in most newborns with a diagnosis of CCAM. It may range in severity from grunting, tachypnea, and a mild oxygen requirement to fulminant respiratory failure requiring aggressive ventilator support or extracorporeal membrane oxygenation (ECMO).

Multiple mechanisms account for the onset of respiratory difficulty. Pulmonary hypoplasia may arise as a consequence of a large CCAM, mediastinal shift may compromise cardiac and respiratory function, spontaneous pneumothoraces may occur, and air trapping within the cyst leads to compression of functional pulmonary tissue.

Recurrent infection: Children in whom the CCAM has not been resected are at risk of recurrent pulmonary infections due to bronchial compression, air trapping, and inability to clear secretions.

Hemoptysis: Hemoptysis has occasionally been described as a manifestation of CCAM in the older child.

Dyspnea and chest pain: Dyspnea may be a feature of pneumothorax, which has been described as a presenting feature of CCAM.

Miscellaneous: Cough, fever, and failure to thrive have all been reported in association with the presentation of CCAM.

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Physical

Generally, the physical signs observed in children with CCAM are nonspecific.

Tachypnea: Tachypnea is the most common sign encountered in the newborn period, reflecting respiratory distress.

Pneumothorax/air trapping: Signs consistent with a pneumothorax or air trapping may be elicited, including tracheal deviation, which indicates mediastinal shift, shifted heart sounds, and decreased air entry on the affected side.

The CT scan of the chest reveals a right lower lobe congenital cystic adenomatoid malformation (CCAM) in a 6-week-old infant who presented with tachypnea. The most striking feature is the solitary enlarged cyst surrounded by numerous microcysts. This lesion was resected without complication.

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo